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List of works by Steven M Hersch

A cell-based assay for aggregation inhibitors as therapeutics of polyglutamine-repeat disease and validation in Drosophila ⬇️

scientific article published on May 13, 2003

A genome scan for modifiers of age at onset in Huntington disease: The HD MAPS study.

scientific article

A novel method for detecting 7-methyl guanine reveals aberrant methylation levels in Huntington disease

scientific article

A small-molecule therapeutic lead for Huntington's disease: preclinical pharmacology and efficacy of C2-8 in the R6/2 transgenic mouse

scholarly article

A systems-level "misunderstanding": the plasma metabolome in Huntington's disease

scientific article

A tale of two factors: what determines the rate of progression in Huntington's disease? A longitudinal MRI study

scientific article

Alterations in brain transition metals in Huntington disease: an evolving and intricate story

scientific article published on July 2012

Altered selenium status in Huntington's disease: neuroprotection by selenite in the N171-82Q mouse model

scientific article published on 8 July 2014

Altered white matter microstructure in the corpus callosum in Huntington's disease: implications for cortical "disconnection".

scientific article published on 19 October 2009

Anti-inflammatory treatment with acetylsalicylate or rofecoxib is not neuroprotective in Huntington's disease transgenic mice

scientific article (publication date: November 2004)

Candidate glutamatergic and dopaminergic pathway gene variants do not influence Huntington's disease motor onset

scientific article

Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease.

scientific article published on September 2002

Cellular localization and development of neuronal intranuclear inclusions in striatal and cortical neurons in R6/2 transgenic mice

scientific journal article

Cerebral cortex and the clinical expression of Huntington's disease: complexity and heterogeneity

scientific article published on 12 March 2008

Chronology of behavioral symptoms and neuropathological sequela in R6/2 Huntington's disease transgenic mice

scientific article published in October 2005

Clinical-Genetic Associations in the Prospective Huntington at Risk Observational Study (PHAROS): Implications for Clinical Trials

scientific article

Common SNP-based haplotype analysis of the 4p16.3 Huntington disease gene region

scientific article

Complexity and heterogeneity: what drives the ever-changing brain in Huntington's disease?

scientific article

Creatine therapy provides neuroprotection after onset of clinical symptoms in Huntington's disease transgenic mice

scientific article

Cystamine increases L-cysteine levels in Huntington's disease transgenic mouse brain and in a PC12 model of polyglutamine aggregation

scientific article published in October 2004

Cysteine oxidation within N-terminal mutant huntingtin promotes oligomerization and delays clearance of soluble protein

scientific article

Cysteine oxidation within N-terminal mutant huntingtin promotes oligomerization and delays clearance of soluble protein

scientific article published in Journal of Biological Chemistry

Cytochrome C and caspase-9 expression in Huntington's disease

scientific article

Demonstration of nondeclarative sequence learning in mice: development of an animal analog of the human serial reaction time task

scientific article published on 10 November 2004

Diffusion tensor imaging in presymptomatic and early Huntington's disease: Selective white matter pathology and its relationship to clinical measures.

scientific article

Discovery of bioactive small-molecule inhibitor of poly adp-ribose polymerase: implications for energy-deficient cells

scientific article published on 01 July 2006

Drug targeting of dysregulated transcription in Huntington's disease

scientific article published on 23 February 2007

Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice

scientific article published in March 2002

Electrocardiogram Abnormalities Suggest Aberrant Cardiac Conduction in Huntington's Disease

Evaluation of histone deacetylases as drug targets in Huntington's disease models. Study of HDACs in brain tissues from R6/2 and CAG140 knock-in HD mouse models and human patients and in a neuronal HD cell model

scientific article

Genome-wide significance for a modifier of age at neurological onset in Huntington's disease at 6q23-24: the HD MAPS study

scientific article

HIP14, a novel ankyrin domain-containing protein, links huntingtin to intracellular trafficking and endocytosis

scientific article (publication date: November 2002)

HTRF analysis of soluble huntingtin in PHAROS PBMCs ⬇️

scientific article published on August 21, 2013

Histone Deacetylase Inhibition by Sodium Butyrate Chemotherapy Ameliorates the Neurodegenerative Phenotype in Huntington's Disease Mice ⬇️

scientific article published on October 15, 2003

Histones associated with downregulated genes are hypo-acetylated in Huntington's disease models

scientific article

Huntington's disease: prospects for neuroprotective therapy 10 years after the discovery of the causative genetic mutation

scientific article

Identification of phenylbutyrate-generated metabolites in Huntington disease patients using parallel liquid chromatography/electrochemical array/mass spectrometry and off-line tandem mass spectrometry

scientific article published on 13 January 2010

Iron accumulates in Huntington's disease neurons: protection by deferoxamine

scientific article

KEAP1-modifying small molecule reveals muted NRF2 signaling responses in neural stem cells from Huntington's disease patients

scientific article

LBH589, A Hydroxamic Acid-Derived HDAC Inhibitor, is Neuroprotective in Mouse Models of Huntington's Disease

scientific article published on December 2016

Linking SNPs to CAG repeat length in Huntington's disease patients.

scientific article published on 19 October 2008

Mechanisms of copper ion mediated Huntington's disease progression

scientific article

Metabolite identification using a nanoelectrospray LC-EC-array-MS integrated system

scientific article published on 25 June 2008

Microtiter plate quantification of mutant and wild-type huntingtin normalized to cell count.

scientific article published on 4 December 2010

Minocycline is protective in a mouse model of Huntington's disease

scientific article published in December 2003

Monitoring reportable events and unanticipated problems: the PHAROS and PREDICT studies of Huntington disease.

scientific article

Motor, cognitive, and functional declines contribute to a single progressive factor in early HD.

scientific article

Neuroprotection for Huntington's disease: ready, set, slow

scientific article published on April 2008

Neuroprotective effects of synaptic modulation in Huntington's disease R6/2 mice.

scientific article

Optimization of an HTRF Assay for the Detection of Soluble Mutant Huntingtin in Human Buffy Coats: A Potential Biomarker in Blood for Huntington Disease

scientific article

PRECREST: a phase II prevention and biomarker trial of creatine in at-risk Huntington disease

scientific article published on 07 February 2014

Population stratification may bias analysis of PGC-1α as a modifier of age at Huntington disease motor onset

scientific article

Reduced creatine kinase as a central and peripheral biomarker in Huntington's disease

scientific article

Resistance of alpha -synuclein null mice to the parkinsonian neurotoxin MPTP.

scientific article

Rho Kinase Pathway Alterations in the Brain and Leukocytes in Huntington's Disease

scientific article published on 5 May 2015

Single-step detection of mutant huntingtin in animal and human tissues: a bioassay for Huntington's disease

scientific article

Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease

scientific article

Sp1 is up-regulated in cellular and transgenic models of Huntington disease, and its reduction is neuroprotective

scientific article published on 4 April 2006

TAA repeat variation in the GRIK2 gene does not influence age at onset in Huntington's disease

scientific article

The CREST-E study of creatine for Huntington disease: A randomized controlled trial

scientific article

The PTB domain of ShcA couples receptor activation to the cytoskeletal regulator IQGAP1

scientific journal article

The Sirtuin 2 microtubule deacetylase is an abundant neuronal protein that accumulates in the aging CNS ⬇️

scientific article (publication date: 15 October 2011)

The mTOR kinase inhibitor Everolimus decreases S6 kinase phosphorylation but fails to reduce mutant huntingtin levels in brain and is not neuroprotective in the R6/2 mouse model of Huntington's disease

scientific article

The sirtuin 2 inhibitor AK-7 is neuroprotective in Huntington's disease mouse models

scientific article published on 29 November 2012

The therapeutic role of creatine in Huntington's disease

scientific article

Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's disease.

scientific article

Therapeutic effects of cystamine in a murine model of Huntington's disease.

scientific article published in October 2002

Transcriptional modulator H2A histone family, member Y (H2AFY) marks Huntington disease activity in man and mouse

scientific article

Translating therapies for Huntington's disease from genetic animal models to clinical trials

scientific article

Using advances in neuroimaging to detect, understand, and monitor disease progression in Huntington's disease

scientific article

Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset

scholarly article

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